Autoimmune Hepatitis Part I
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Autoimmune hepatitis (AIH) is an immune-mediated inflammatory liver disease characterized by elevated liver enzymes, positive autoantibodies, and interface hepatitis on liver biopsy. It can present in various ways, including asymptomatically, with acute hepatitis, or with cirrhosis. Genetic susceptibility and environmental triggers like viruses are thought to play a role in breaking immune tolerance against liver antigens. This leads to an immune response involving autoantibodies, cytotoxic T cells, and inflammatory cytokines that damage liver cells. Treatment involves immunosuppression with corticosteroids and azathioprine to induce and maintain remission.
![Autoimmune Hepatitis, 2020. Ayman Alsebaey, MD 39
Features Type 1 AIH Type 2 AIH
Frequency US adults, 96%
US children, 9%‐12%
UK children, 38%
Age at presentation Peripubertal and adults Usually under 14 years
Mode of presentation
Chronic symptoms common Acute onset (~40%)
Ascites or GI bleeding rare Acute liver failure possible
Asymptomatic in 25%‐34% Relapse frequent
Acute in 25%‐75%
Acute severe in 2%‐6%
Laboratory features Hypergammaglobulinemia IgA levels may be reduced
Autoantibodies
ANA Anti‐LKM1
SMA, anti‐actin [Anti‐LC1, Anti‐LKM3]
SLA
Concurrent immune diseases
Autoimmune thyroiditis Autoimmune thyroiditis
Rheumatic diseases Diabetes mellitus
IBD Vitiligo
Autoimmune overlap with PSC (ASC
in children)
Common in children Rare
Atypical pANCA‐positive Atypical pANCA‐negative
Overlap with PBC Seen in adults (not children) Not reported
Cirrhosis at presentation
Adults, 28%‐33% (especially elderly)
Rare
Children, ≤33%
Remission after drug withdrawal Possible Rare, usually need long‐term immunosuppression](https://image.slidesharecdn.com/autoimmunehepatitisparti-200807201648/85/Autoimmune-Hepatitis-Part-I-39-320.jpg)
Autoimmune Hepatitis Part I
- 1. Autoimmune Hepatitis Part I Ayman Alsebaey, MD Associate Professor of Hepatology and Gastroenterology National Liver Institute, Menoufia University, Egypt June 2020
- 2. ﷽ .....ا َي ْن ُّالد ي ِف ا َن ِآت ا َن َّب َر ِق َو ًة َن َس َح ِة َر ِخ ْاْل ي ِف َو ًة َن َس َحَاب َذ َع ا َن ِار َّالن Autoimmune Hepatitis, 2020. Ayman Alsebaey, MD 2
- 3. Agenda Definition. Pathogenesis Clinical picture. Investigations. Classification. Autoimmune Hepatitis, 2020. Ayman Alsebaey, MD 3
- 4. AIH Definition Autoimmune Hepatitis, 2020. Ayman Alsebaey, MD 4
- 5. • AIH was first described in 1951 by Waldenström then was coined lupoid hepatitis. • There is loss of tolerance against liver antigens that is triggered by environmental factors in individuals with a certain genetic susceptibility. • It is immune mediated inflammation of the liver associated with: • Elevated AST and ALT. • Positive antibodies. • High IgG levels. • Interface hepatitis in liver biopsy. • ± associated other system autoimmune diseases. Autoimmune Hepatitis, 2020. Ayman Alsebaey, MD 5
- 6. Epidemiology • AIH occurs globally in children and adults of all ages and in all ethnicities. • Alaskan Natives: icteric AIH • Hispanics: cirrhosis • African Americans: accelerated progression and a higher rate of recurrence after LT. • Age onset: • From first year of age to age of 80. • Commonly 2 peaks (10-18y), (40y) and rarely >60y. • Sex: • Female predilection (4:1). • Family History: • In half of the patients there is family history of autoimmune diseases. Autoimmune Hepatitis, 2020. Ayman Alsebaey, MD 6 20% 80% 0% 10% 20% 30% 40% 50% 60% 70% 80% 90% Male Female
- 7. AIH Pathogenesis Autoimmune Hepatitis, 2020. Ayman Alsebaey, MD 7
- 8. AIH is the loss of tolerance against liver antigens. Autoimmune Hepatitis, 2020. Ayman Alsebaey, MD 8 Genetic susceptibility Environmental trigger Immune pathway activation Antibody formation and cell destruction Loss of inhibitory pathway
- 9. Genetics • HLA (MHC) presents antigens to CD4+ T lymphocytes. Autoimmune Hepatitis, 2020. Ayman Alsebaey, MD 9 HLA I HLA II CD4+
- 10. Genetics THERE IS ALTERNATION IN THIS GENE. • DRB1*0301 and DRB1*0401 in European and North American populations (AIH 1). • DRB1*0404 and DRB1*0405 in Japan, Argentina and Mexico(AIH 1). • DRB1*0701 (AIH 2) aggressive disease. • DRB1*0301 (AIH 2) in south America HAV and AIH2. Age Males TTT response Other Dis HLA-DR3 HLA-DR4 Autoimmune Hepatitis, 2020. Ayman Alsebaey, MD 10 • Egypt: HLADRB1*1301, HLADRB1*15 • Other genes: CARD10, CTLA-4, TNFA*2
- 11. Environmental factors • Environmental factors as viruses (molecular mimicry) may trigger AIH. • Dysbiosis: • Dysbiosis is alterations in the composition of the intestinal microbiota • diversity and reduced total load of gut bacteria. • presence of anaerobic bacteria in the gut • gut permeability and increased translocation of intestinal microbial products into the systemic circulation Autoimmune Hepatitis, 2020. Ayman Alsebaey, MD 11
- 12. Molecular mimicry • It is more obvious in patients with AIH2. • HCV, HBV, HAV, EBV, CMV, HSV target P450 2D6 (CYP2D6) anti- LKM1 • HCV +ve ANA, ASMA, LKM. • 50% of AIH2 patients are positive for anti-HCV antibody. • Drugs +ve ANA • Nitrofurantoin, minocycline, statins, adalimumab and infliximab. Autoimmune Hepatitis, 2020. Ayman Alsebaey, MD 12 Floreani et al. Etiopathogenesis of autoimmune hepatitis. Journal of Autoimmunity 2018; 95:133-43.
- 13. Auto antigens • Cytoplasmic antigens are auto- antigens. • CYP2D6 autoantigen +ve LKM1 in AIH and CHC. • Transfer ribonucleoprotein complex tRNP(Ser)Sec autoantigen +ve anti-SLA antibody. • Formimino-transferase cyclodeaminase autoantigen +ve anti-LC1. • UGT autoantigen +ve anti-LKM3. • CYP1A2 AIH + APECED syndrome. • Autoimmune polyendocrinopathy- candidiasis ectodermal dystrophy (APECED) syndrome is AR. • Hypoparathyroidism, primary adrenocortical failure, chronic mucocutaneous candidiasis • CYP1A2 AIH induced by dihydralazine. Autoimmune Hepatitis, 2020. Ayman Alsebaey, MD 13
- 14. Immune cells Mieli-Vergani et al. Autoimmune hepatitis. Nature Reviews Disease Primers 2018; 4:18017.Autoimmune Hepatitis, 2020. Ayman Alsebaey, MD 14 APC, antigen-presenting cell; CTL, cytotoxic CD8+ T lymphocyte; Fc, crystallizable fragment; IFNγ, interferon-γ; MHC, major histocompatibility complex; NK, natural killer; TCR, T cell receptor; TFH, T follicular helper; TGFβ, transforming growth factor- β; TH0, naive CD4+ T helper; TH1, T helper 1; TH2, T helper 2; TH17, T helper 17; TNF, tumour necrosis factor; Treg, regulatory T. CD8+ CD4+ DCs, macrophages, B cells LSEC, Kupffer cells Act as APCs CYP2D6 Granzyme B IFNγ CD4+CD25+ CD4+CD25+CD127−cells CD4+CD25+CD127−FOXP3+ IgG–secretingplasmacells Gamma delta (γδ) T cells
- 15. Antibodies • They are detected by indirect immunofluorescence. • They are positive in many diseases • They help in AIH diagnosis. • They help in AIH classification. • They have no role in decision of treatment or monitoring of therapy. • The titers decrease with treatment and increase with flare or relapse. • Anti-nuclear antibody (ANA) • Smooth muscle antibody (ASMA) • Anti-liver kidney microsomal antibody (LKM) • ANTI-LM, ANTI-LKM-2, 3 • Anti-liver cytosol type 1 (anti-LC1) • Anti-soluble liver antigen/liver–pancreas antigen (Anti-SLA/anti-LP) • Anti-neutrophil cytoplasmic antibodies (ANCA). • Anti-chromatin, anti dsDNA, anti-CCP, anti-ASGPR. Autoimmune Hepatitis, 2020. Ayman Alsebaey, MD 15
- 16. Autoimmune Hepatitis, 2020. Ayman Alsebaey, MD 16 ANA LKM1 ASMA LC1 ANA ANA AMA Terziroli et al. The clinical usage and definition of autoantibodies in immune-mediated liver disease: A comprehensive overview. Journal of Autoimmunity 2018; 95:144-58.
- 17. Antibodies Autoimmune Hepatitis, 2020. Ayman Alsebaey, MD 17 Target Disease association ANA Multiple nuclear antigens AIH, SLE, PBC, PSC, drug hepatitis, alcoholic liver disease and viral hepatitis. AMA 2-oxo-acid-dehydrogenase complex PBC pANCA h-Lamp-2, proteinase 3 AIH, PSC, PBC ASMA Actin, troponin, tropomyosin AIH I, infectious and rheumatic disorders VGT pattern: earlier disease LKM 1 CYP 2D6 AIH II, HCV LKM 2 CYP 2C9 Tienilic acid-induced hepatitis LKM 3 UGT1A AIH, hepatitis D LM CYP 2A6 APECED, hepatitis C, Dihydralazine induced hepatitis LC1 FTCD AIH II or HCV SLA/LP tRNP(Ser)Sec AIH I and II, PSC LM CYP 1A2 Dihydralzine-induced hepatitis, APECED ASGP-R Asialoglycoprotein receptor Autoimmune liver disease, HCV ANA, anti-nuclear antibodies; AMA, anti-mitochondrial antibodies; ANCA, antineutrophilic cytoplasmatic antibodies; SMA, smooth muscle antibodies; LKM, liver kidney microsomal antibodies; LM, liver microsomal antibodies; LC1, liver cytosolic antibodies type 1; SLA/LP, soluble liver antigen/liver pancreas antibodies; ASGPR-R, asialoglycoprotein receptor antibodies; UGT1A, UDP glucuronosyltransferase family 1 A; FTCD, formimino-transferase cyclodeaminase; AIH, autoimmune hepatitis; PSC, primary sclerosing cholangitis; PBC, primary biliary cirrhosis; HCV, hepatitis C virus; APECED, autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy.
- 18. New Antibodies • Anti-huntingtin-interacting protein 1-related protein (anti-HIP1R protein) antibodies: • They are elevated in AIH patients. • They have a higher specificity than and equal sensitivity to ANA and SMA Autoimmune Hepatitis, 2020. Ayman Alsebaey, MD 18
- 19. 49% 51% 48% 49% 49% 50% 50% 51% 51% 52% Isolated Multiple Antibodies Autoimmune Hepatitis, 2020. Ayman Alsebaey, MD 19 80% 63% 3% 0% 20% 40% 60% 80% 100% ANA ASMA LKM1 At presentation 29% 26% 32% 34% 21% 0% 5% 10% 15% 20% 25% 30% 35% 40% PSC HCV HBV NAFLD ALD Isolated ANA 6% 6% 4% 0% 1% 2% 3% 4% 5% 6% 7% PSC HCV ALD Isolated ASMA
- 20. Clinical Presentations Autoimmune Hepatitis, 2020. Ayman Alsebaey, MD 20
- 21. AIH Presentations Autoimmune Hepatitis, 2020. Ayman Alsebaey, MD 21 Asymptomatic Acute hepatitis Cirrhosis Fulminant Liver Other phenotypes Antibody –ve Overlap syndrome DI-AIH De novo Post TX APECED
- 22. AIH Presentations • Asymptomatic (25%) • They are discovered accidently. • 10% spontaneous laboratory improvement. • They develop symptoms within 32months. • Less survival than treated patients with more severe disease (67% versus 98%). • Acute hepatitis: • Patients may present with hepatitis. • Fatigue, malaise, or amenorrhea • Arthralgia is characteristic. • Liver biopsy may reveal cirrhosis or advanced fibrosis. • Cirrhosis: • AIH is detected by investigations for cirrhosis. • 30% of AIH patients have cirrhosis at presentation • 30% are decompensated. Autoimmune Hepatitis, 2020. Ayman Alsebaey, MD 22
- 23. AIH Presentations • Acute liver cell failure “fulminant”: • It is very difficult to diagnosis AIH in this setting. • Antibodies and IgG may be negative. • Transjugular liver biopsy: centrilobular lesions and necrosis . • Associated autoimmune diseases: • They occur later one and few cases have simultaneous diseases. • Other phenotypes or presentations. Autoimmune Hepatitis, 2020. Ayman Alsebaey, MD 23
- 24. One Face or Multiple Faces Disease Autoimmune Hepatitis, 2020. Ayman Alsebaey, MD 24
- 25. Wang et al. The clinical phenotypes of autoimmune hepatitis: A comprehensive review. Journal of Autoimmunity 2016; 66:98-107.Autoimmune Hepatitis, 2020. Ayman Alsebaey, MD 25 Clinical phenotypes Characteristic features Diagnostic and treatment strategies Autoantibody- negative AIH -ve ANA, ASMA, LKM-1 and/or AMA. use revised criteria (1999) for diagnosis. Same response to steroids. DI-AIH Drugs trigger AIH. Rare post steroids relapse. Stop offending drug and avoid re- exposure. APECED Addison's disease, hypoparathyroidism and mucocutaneous candidiasis rare variant of AIH occurs in 10%–18% of patients with APECED AIH-PBC overlap Concurrent features of PBC The Paris Criteria (1998) Steroids + UDCA AIH-PSC overlap Concurrent features of PSC Require MRCP and/or ERCP Steroids + AZA +UDCA Recurrent AIH post- transplantation Absent of the acute rejection histological features. Steroids + AZA De novo AIH Development of AIH after LT for other liver diseases Prompt AIH standard regimens combined with a low dose of calcineurin inhibitor
- 26. Autoimmune Hepatitis, 2020. Ayman Alsebaey, MD 26 Autoimmune Polyendocrinopathy Candidiasis Ectodermal Dystrophy Kisand et al. Autoimmune Polyendocrinopathy Candidiasis Ectodermal Dystrophy. Journal of Clinical Immunology 2015; 35:463-78.
- 27. Uveitis T1DM Thyroiditis ITP AIHA Glomerulonephritis Autoimmune Hepatitis, 2020. Ayman Alsebaey, MD 27 AIH Associated Diseases Celiac disease, IBD RA, SLE, CREST, Sjogren, systemic sclerosis. Vitiligo, Alopecia, Lichen planus, Nail dystrophy
- 28. Autoimmune Hepatitis, 2020. Ayman Alsebaey, MD 28 Type 1 AIH Type 2 AIH Concurrent immune diseases Autoimmune thyroiditis Autoimmune thyroiditis Rheumatic diseases Diabetes mellitus IBD Vitiligo 0% 5% 10% 15% 20% 25% 30% 35% 40% 45% Autoimmune Thyroid Rheumatic Disease IBD Hemolytic Anemia ≤30 years ≥60 years
- 29. Investigations Autoimmune Hepatitis, 2020. Ayman Alsebaey, MD 29
- 30. There is no single pathognomonic test for AIH it is a disease of exclusion • LFTs: • Acute hepatitis: AST, ALT, Bilirubin, ± ALP. • Chronic hepatitis: AST, ALT. No correlation with histopathology. • Cirrhosis: AST, ALT, Bilirubin. • ?AST >ALT. • If Bilirubin indirect >direct search for autoimmune hemolytic anemia. • CBC: • Normal in early disease. • Platelets with cirrhosis. • Hb search for autoimmune hemolytic anemia. • INR: • Prolonged in cirrhosis or overlap syndrome. • Viral Profile: • Negative for HBV, HCV, HEV, EBV, CMV and others. • Antibodies: • +ve ANA, ASMA,LKM according to AIH type. • -ve AMA. • Metabolic profile: • -ve Wilson, Hemochromatosis. Autoimmune Hepatitis, 2020. Ayman Alsebaey, MD 30
- 31. Investigations • IgG • IgG with normal IgA and IgM. • Helps the diagnosis and monitoring of therapy. • IgG Caveats: • Wide range. • 85% IgG. • 15% normal IgG.! • Is it normal or increased level in a patients with physiological low level. • Imaging: • Normal abdominal US in early stages. • Cirrhosis features in advanced stages. Autoimmune Hepatitis, 2020. Ayman Alsebaey, MD 31 Wide IgG range
- 32. Investigations: LIVER BIOPSY • Histopathology: • It is mandatory for the diagnosis, exclusion of other disease, monitoring of the disease and response to treatment. • Interface hepatitis: • a lymphoplasmacytic infiltrate which crosses the limiting plate. • It occurs with AIH, HCV and DILI. • Plasma cells may be seen panlobular panlobular hepatitis. • Emperipolesis: • Penetration of one intact cell into another intact cell, with both cells retaining viability (as opposed to phagocytosis) lymphocytes within hepatocytes. • Hepatocytes: • Swollen or pyknotic. • Dying cells are surrounded by lymphocytes, plasma cells and histiocytes. • Cirrhosis: macronodular type. • Hepatocyte rosette formation. Autoimmune Hepatitis, 2020. Ayman Alsebaey, MD 32 66% 47% 29% 65% 33% 40% 20% 0% 10% 20% 30% 40% 50% 60% 70% Interface Hepatitis Lobular Hepatitis Centrilobular Necrosis Emperipolesis Hepatocyte Rosettes Cirrhosis AIH-NASH
- 33. Wang et al. The clinical phenotypes of autoimmune hepatitis: A comprehensive review. Journal of Autoimmunity 2016; 66:98-107. Autoimmune Hepatitis, 2020. Ayman Alsebaey, MD 33 (A) Moderate interface hepatitis (C) Hepatocyte rosette (B) Lymphocyte and plasma cell infiltration (D) Emperipolesis
- 34. Histological activity index for chronic hepatitis Autoimmune Hepatitis, 2020. Ayman Alsebaey, MD 34 HAI 1-3: minimal hepatitis or remission. HAI 4-8: mild hepatitis. HAI 9-12: moderate hepatitis. HAI 13-18: severe hepatitis
- 35. Noninvasive Fibrosis Assessment • Vibration‐Controlled Transient Elastography (VCTE): • FibroScan is useful. • In the 1st 3-6 months of treatment: reflection of the inflammation >fibrosis due to ATs. • After 6 months of treatment: reflection of liver fibrosis. • F ≥2: 5.8 kPa • F ≥3: 10.5 kPa • F ≥4: 16 kPa • VCTE correlate with biochemical remission, regression of fibrosis, and favorable prognosis when assessed after 6 months of treatment. • Magnetic resonance elastography (MRE) • It should be done after 6 months of treatment. • Acoustic radiation force impulse imaging (ARFI) • Further studies are needed. Autoimmune Hepatitis, 2020. Ayman Alsebaey, MD 35
- 36. AIH Classifications Autoimmune Hepatitis, 2020. Ayman Alsebaey, MD 36
- 37. AIH Classification Autoimmune Hepatitis, 2020. Ayman Alsebaey, MD 37
- 38. Recent AIH Classification • Type I: • +ve ANA and/or ASMA and/or anti-SLA. • Type II: • +ve anti-LKM-1 and/or anti-LC1 and/or anti-LKM-3. • It is common in children and 5% of adults. • Antibodies negative AIH: • -ve antibodies. 80% 20% 0% 10% 20% 30% 40% 50% 60% 70% 80% 90% AIH I AIH II Autoimmune Hepatitis, 2020. Ayman Alsebaey, MD 38
- 39. Autoimmune Hepatitis, 2020. Ayman Alsebaey, MD 39 Features Type 1 AIH Type 2 AIH Frequency US adults, 96% US children, 9%‐12% UK children, 38% Age at presentation Peripubertal and adults Usually under 14 years Mode of presentation Chronic symptoms common Acute onset (~40%) Ascites or GI bleeding rare Acute liver failure possible Asymptomatic in 25%‐34% Relapse frequent Acute in 25%‐75% Acute severe in 2%‐6% Laboratory features Hypergammaglobulinemia IgA levels may be reduced Autoantibodies ANA Anti‐LKM1 SMA, anti‐actin [Anti‐LC1, Anti‐LKM3] SLA Concurrent immune diseases Autoimmune thyroiditis Autoimmune thyroiditis Rheumatic diseases Diabetes mellitus IBD Vitiligo Autoimmune overlap with PSC (ASC in children) Common in children Rare Atypical pANCA‐positive Atypical pANCA‐negative Overlap with PBC Seen in adults (not children) Not reported Cirrhosis at presentation Adults, 28%‐33% (especially elderly) Rare Children, ≤33% Remission after drug withdrawal Possible Rare, usually need long‐term immunosuppression
- 40. AIH patients without elevation of IgG • 10% of all patients with AIH. • Indistinguishable from patients with typical AIH by biochemical markers or liver histology. • They have no selective IgG elevation, with lower IgG and IgA levels than patients with typical AIH. • Stable remission off treatment >typical AIH group. • These patients might represent a subgroup in whom there is a high chance of successful drug withdrawal. Autoimmune Hepatitis, 2020. Ayman Alsebaey, MD 40 Hartl et al. Features and outcome of AIH patients without elevation of IgG. JHEP Reports 2020; 2:100094.
- 41. AIH Diagnosis Autoimmune Hepatitis, 2020. Ayman Alsebaey, MD 41
- 42. AIH Diagnosis • As with other autoimmune diseases, the diagnosis is a labyrinth. • DIAGNOSIS OF EXCLUSION. • NO DIAGNOSTIC TEST TILL NOW. • INTERNATIONAL SCORES WERE PUT TO HELP DIAGNOSIS. Autoimmune Hepatitis, 2020. Ayman Alsebaey, MD 42
- 43. AIH Diagnosis AIH AST, ALT IgG +ve Histopathology Exclude other diseases +ve Family history Other autoimmune diseases Suggestions Autoimmune Hepatitis, 2020. Ayman Alsebaey, MD 43
- 44. REVISED International Autoimmune Hepatitis Group diagnostic (IAIHG) scoring system (1999) Autoimmune Hepatitis, 2020. Ayman Alsebaey, MD 44 Parameter Feature Score Principal parameters Sex Female +2 ALP:AST (or ALT) ratio >3 −2 1.5–3 0 <1.5 +2 Serum globulins or IgG (times above normal) >2.0 +3 1.5–2.0 +2 1.0–1.5 +1 <1.0 0 ANA, SMA, or anti-LKM-1 titers >1:80 +3 1:80 +2 1:40 +1 <1:40 0 AMA Positive −4 Viral markers of active infection Positive −3 Negative +3 Hepatotoxic drug history Yes −4 No +1 Average alcohol <25 g/day +2 >60 g/day −2
- 45. Revised International Autoimmune Hepatitis Group diagnostic (IAIHG) scoring system (1999) Autoimmune Hepatitis, 2020. Ayman Alsebaey, MD 45 Parameter Feature Score Principal parameters Histological features Interface hepatitis +3 Plasma cells +1 Rosettes +1 None of above −5 Biliary changesa −3 Atypical changesb −3 Optional additional parameters Seropositivity for other defined autoantibodies Anti-SLA/LP, actin, LC1, ASGPR, p-ANCA +2 HLA DR3 or DR4 +1 Response to therapy Remission +2 Relapse +3 Interpretation of aggregate scores Pre-treatment Definite AIH >15 Probable AIH 10–15 Post-treatment Definite AIH >17 Probable AIH 12–17
- 46. Simplified criteria for the diagnosis of autoimmune hepatitis (2008) Autoimmune Hepatitis, 2020. Ayman Alsebaey, MD 46 Variable Cut-off Points ANA or SMA ≥1:40 +1 ANA or SMA ≥1:80 +2 Or Anti-LKM1 (alternative to ANA and SMA) ≥1:40 +2 Or Anti-SLA (alternative to ANA, SMA and LKM1) Positive +2 IgG or γ-globulins level >ULN +1 >1.10 times ULN +2 Liver histology Compatible with AIH +1 Typical of AIH +2 Atypical 0 Absence of viral hepatitis Yes +2 ≥6 probable AIH ≥7 definite AIH Less reliable in children and atypical groups of AIH patients (seronegative disease, normal IgG levels) and those with co-existing chronic viral hepatitis
- 47. Autoimmune Hepatitis, 2020. Ayman Alsebaey, MD 47 Typical and compatible histology for the diagnosis of AIH in the 2008 simplified criteria Diagnostic category Histological features Typical AIH: 2 points The three following features showed be present: Interface hepatitis with lymphocytic or lymphoplasmacytic portal inflammatory infiltrates extending into the lobule Hepatocyte rosette formation Emperipolesis Compatible with AIH: 1 point Chronic hepatitis with lymphocytic infiltration without all the features considered typical Atypical 0 points Evidence of another diagnosis
- 48. • Revised diagnostic criteria (1999): • More accurate than the simplified diagnostic criteria for diagnosis of AIH in patients with complex medical histories of comorbid diseases, multiple medications or alcohol use • The simplified diagnostic criteria (2008): • They are preferred for patients with typical biochemical, serological and histological features of AIH. Autoimmune Hepatitis, 2020. Ayman Alsebaey, MD 48 100% 73% 82% 95% 90% 92% 60% 65% 70% 75% 80% 85% 90% 95% 100% 105% Sensitivity Speceficity Accuracy Revised Simplified
- 49. Limitations of the revised original and simplified scoring systems • Lack of validation by prospective studies • Lack of accuracy in the setting of concurrent PSC, PBC, NAFLD/NASH, LT, or fulminant liver failure • Failure to include other serological markers, such as anti‐SLA • Dependence on autoantibody determinations by indirect immunofluorescence (titers) rather than by enzyme‐linked immunoassay (units). Autoimmune Hepatitis, 2020. Ayman Alsebaey, MD 49
- 50. Autoimmune Hepatitis, 2020. Ayman Alsebaey, MD 50 2019
- 51. ﷽ ......ِإ َت ْل َنز َأ ا َم ِل ِّين ِإ ِّب َرَّي َل ٌير ِق َف ٍر ْي َخ ْن ِم Autoimmune Hepatitis, 2020. Ayman Alsebaey, MD 51
- 52. Thanks a lot Autoimmune Hepatitis, 2020. Ayman Alsebaey, MD 52